GLYCOGEN DISEASE IN CHILDREN: THE PROBLEM OF DIAGNOSIS AND TREATMENT


A.N. Surkov

FSBI Scientific Center for Children's Health, Moscow
Despite significant advances in domestic medicine, whole group of inherited disorders of carbohydrate metabolism, under the general term "glycogen disease" continues to remain underdiagnosed in our country. These disorders are characterized by excessive accumulation of normal or restructured glycogen in various organs and tissues, most commonly in the liver and muscles. Pathogenetic therapy for hepatic forms of glycogen disease (GD) has not yet been developed. All this inevitably entails early disability of patients and significantly increases the risk of death from complications incompatible with life. However, strict adherence to a specialized diet, symptomatic therapy and effective self-control allow to avoid metabolic decompensation, which significantly improves the prognosis of the disease and improves the quality of life of patients. In this connection, constant improvement in terms of medical diagnostics and therapy of GB in children is necessary. The article presents the generalize information on these issues and discusses the most relevant directions for further research.

About the Autors


A.N. Surkov– FSBI Scientific Center for Children's Health, Moscow


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